Complement C4

From Proteopedia

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spinqualitylabelsall models Glycosylated human complement C4-A α chain (grey), C4 β (green) and γ chain (pink) (PDB code 4fxk) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image Function Complement C4 (CC4) is part of the complement pathway. CC4 is a mixture of CC4-A and CC4-B. CC4 is part of the complement cascade which results in pathogen cell membrane pore and the eventual lysis of the pathogen cell[1]. Disease C4 deficiency causes lupus-like autoimmunity[2]. Copy number variations of complement C4 are associated with Behcet's disease[3]. Level of complement C4 is associated with persistence and disease progression of chronic hepatitis C[4].

3D structures of complement C4

Updated on 15-May-2019

5jpn, 5jtw – hCC4-A - human
1hzf – hCC4-A residues 970-1336
5jpm – hCC4-A + mannan-binding lectin serine protease
4xam – hCC4-A residues 757-1744 + hCC4-B residues 20-675
4fxk – hCC4-A α chain residues 680-1446 + hCC4 β residues 2—675 +γ residues 1454-1744 chains
4fxg – hCC4-A α chain residues 680-1446 + hCC4 β residues 2—675 +γ residues 1454-1744 chains + mannan-binding lectin serine protease

References

1. ↑ Nesargikar PN, Spiller B, Chavez R. The complement system: history, pathways, cascade and inhibitors. Eur J Microbiol Immunol (Bp). 2012 Jun;2(2):103-11. doi:, 10.1556/EuJMI.2.2012.2.2. Epub 2012 Jun 13. PMID:24672678 doi:http://dx.doi.org/10.1556/EuJMI.2.2012.2.2
2. ↑ Chen Z, Koralov SB, Kelsoe G. Complement C4 inhibits systemic autoimmunity through a mechanism independent of complement receptors CR1 and CR2. J Exp Med. 2000 Nov 6;192(9):1339-52. PMID:11067882
3. ↑ Hou S, Qi J, Liao D, Zhang Q, Fang J, Zhou Y, Liu Y, Bai L, Zhang M, Kijlstra A, Yang P. Copy number variations of complement component C4 are associated with Behcet's disease but not with ankylosing spondylitis associated with acute anterior uveitis. Arthritis Rheum. 2013 Nov;65(11):2963-70. doi: 10.1002/art.38116. PMID:23918728 doi:http://dx.doi.org/10.1002/art.38116
4. ↑ Chowdhury SJ, Karra VK, Bharali R, Kar P. Role of complement component C4 in treatment response and disease progression in chronic hepatitis C patients. J Viral Hepat. 2015 Aug;22(8):671-4. doi: 10.1111/jvh.12383. Epub 2015 Jan 9. PMID:25573496 doi:http://dx.doi.org/10.1111/jvh.12383